Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In summary, Wegener's granulomatosis is a systemic illness that is characterized pathologically by necrotizing granulomatosis and vasculitis. Lethal midline granuloma is not a pathological term. Lethal midline granuloma is a clinical term, and in reality, lethal midline granuloma has been proven to be polymorphic reticulosis and not Wegener's granulomatosis. Polymorphic reticulosis is now understood to be a T-cell lymphoma. Further study of these malignant lymphomas has shown that these T-cell lymphoproliferative disorders may be causally associated with Epstein-Barr virus. Although there is strong association between autoantibodies against cytoplasmic constituents of neurophils and monocytes in patients with active Wegener's granulomatosis, the exact pathogenic mechanism in Wegener's granulomatosis and the etiology is still unknown. IgG-C-ANAC (C-ANAC stands for Classical/Cytoplasmic Anti-Neutrophil Cytoplasm Antibodies) increases with increasing disease activity from undetectable levels to up to 95% of active Wegener's granulomatosis patients. Rhinologic symptoms in Wegener's granulomatosis include progressive nasal obstruction, bloody rhinorrhea with crusting, and vague pain and tenderness of the nasal dorsum. Usually these patients have mucosal ulcerations of the nose with or without a septal perforation. Other areas of the head and neck can be involved, and they include orbital, otologic, oral, and subglottic involvement. Hallmarks of malignant lymphoma (polymorphic reticulosis) when it involves the upper airway include rapid localized destruction of the nose, orbit, paranasal sinuses, and hard palate. Treatment for Wegener's granulomatosis includes antimicrobial agents in addition to a regimen of cyclophosphamide and glucocorticoids. The treatment for malignant lymphoma (polymorphic reticulosis) is primarily radiation, especially when confined to one site.
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PMID:Fireside conference 19. Wegener's granulomatosis and lethal midline granuloma. 138 98

Benign lymphocytic angiitis and granulomatosis is a T-cell lymphoproliferative disorder confined to the lung and corresponding to a low-grade angiocentric immunoproliferative lesion. Controversy remains as to whether these lesions are lymphomas. We report such a case in an 8-year-old patient with Burkitt's lymphoma in remission who presented with persistent bronchopneumopathy and bilateral pulmonary infiltrates on tomodensitometry. Surgical resection revealed the histologic changes of benign lymphocytic angiitis and granulomatosis. Immunohistochemistry showed no aberrant pan T-cell marker loss. Genetic analysis of frozen tissue by Southern blot DNA hybridization with probes to T-cell receptor beta- and gamma-chain genes and to the immunoglobulin heavy chain joining region gene (JH) identified no clonal rearrangement. Search for Epstein-Barr virus-DNA sequences by in situ hybridization and Southern blot analysis provided negative results. Our data imply that lowgrade angiocentric immunoproliferative lesions are not exclusively lymphomas but might represent a borderline lymphoproliferative disease (seen in the course of many diseases), perhaps corresponding to host immune response.
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PMID:Benign lymphocytic angiitis and granulomatosis: a T-cell lymphoma? 139 50

Patients with X-linked lymphoproliferative (XLP) disease are characterized by extreme vulnerability to Epstein-Barr virus (EBV). Following infection with EBV, affected males develop fatal infectious mononucleosis (IM), hypogammaglobulinemia (H), or non-Hodgkin's lymphoma (NHL). In addition, hyper IgM, red cell aplasia, necrotizing lymphoid vasculitis (NLV), and aplastic anemia occur rarely. The recent use of DNA restriction fragment length polymorphism (RFLP) probes in linkage with the XLP gene now permit detection of affected males prior to primary EBV infection. We have measured immunoglobulin class and subclass levels in sera from EBV-negative males who were either positive or negative for the XLP genotype by RFLP analysis. Elevated IgA or IgM and/or variable deficiency of IgG, IgG1, and IgG3 occurred in the sera of 13/13 RFLP-positive, EBV-negative males. No consistent abnormalities were noted in 14 RFLP-negative, EBV-negative males. We conclude that the immune defect in XLP is not solely EBV-specific, although EBV is responsible for most of the morbidity and all of the mortality. Further, serial measurement of Ig levels may provide information regarding status of EBV-negative males at risk where RFLP analysis is uninformative or in families where sporadic cases of fatal IM, acquired hypogammaglobulinemia or NHL have occurred, but wherein the genotype of XLP cannot be documented.
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PMID:Immunoglobulin class and subclass deficiencies prior to Epstein-Barr virus infection in males with X-linked lymphoproliferative disease. 168 54

A two-year-old girl with measles virus (MV) and chronic Epstein-Barr virus (EBV) infection developed lethal coronary aneurysmal arteritis accompanied by giant cell pneumonia, systemic lymphadenitis and hepatosplenomegaly. In her coronary arteries, lungs and aorta, cells containing intranuclear and intracytoplasmic inclusions, including syncytial giant cells, were detected, the presence of MV in the organs being proved by electron microscopic and immunofluorescent studies. Immunopathology further demonstrated MV to be disseminated in almost all organs other than lymph nodes. Clinical diagnosis of chronic EBV infection was established on the basis of persistent high titers of antibodies against capsid and early antigens of EBV and viral presence was confirmed by Southern blot hybridization in a mesenterial lymph node obtained at autopsy. To the best of our knowledge, this is the first description of MV association with coronary aneurysmal arteritis, raising the possibility that measles infection can cause severe vasculitis under immuno-suppressive states, such as that caused by chronic EBV infection.
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PMID:Measles associated with coronary arteritis. 196 61

The possible association of Epstein-Barr Virus (EBV) with uveitis was investigated in 28 subjects with various types of uveitis. Serum or aqueous humor (AH) EB viral antibodies were measured in 28 uveitis patients and in 28 cataract patients as controls. Significant rises in EBV antibody levels were seen in 3 cases from the uveitis group. There was an 8-fold increase of the serum antibody titer in an infant subject, a 16-fold increase in the AH EB viral antibody titer in an adult subject, and a higher antibody quotient (10.2) compared with the serum and AH antibody titers in another adult case. No antibodies to EBV were detected in the AH in the control group. It appeared that EBV might have been involved in the pathogenesis of these 3 cases of uveitis. The clinical features of these cases were as follows. (1) A common cold-like prodrome was noted. (2) Severe acute anterior uveitis with fibrinous exudate was present at the onset. (3) A transition to chronic granulomatous anterior uveitis was seen in the 2 adult cases. (4) Little vitreous inflammation was present. (5) Marked hyperemia and swelling of the optic discs was noted. (6) At the onset there were no abnormal chorioretinal findings, except slight retinal vasculitis in the infant case. (7) Sunset-glow fundus appeared earlier in comparison with Harada disease in the adults and a retinal pigment epithelium disorder developed in the infant. (8) Bilateral involvement was seen in adults. (9) Good final visual acuity was recognized. (10) No systemic changes occurred in the course of the uveitis. This constellation of clinical features appears to comprise a new entity, i.e., EBV-associated uveitis.
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PMID:[The possible association of Epstein-Barr virus and uveitis]. 217 6

The lesions known as lymphocytic vasculitis, polymorphic reticulosis (midline malignant reticulosis, lethal midline granuloma), lymphomatoid granulomatosis, and angiocentric lymphoma form what have been collectively termed the angiocentric immunoproliferative lesions (AIL). Because of recent reports demonstrating clonal rearrangements of the T-cell receptor in these lesions, the AIL are now thought to represent a continuous spectrum of post-thymic T-cell non-Hodgkin's lymphoma (NHL). NHL associated with the acquired immune deficiency syndrome (AIDS) represents intermediate or high-grade B-cell malignancies in HIV-infected patients that may be etiologically related to the Epstein-Barr virus (EBV). There have been reports of EBV-associated T-cell NHL, AIL, and large granular lymphocyte (LGL) proliferations, as well as HIV-associated T-cell neoplasia, LGL/T-cell proliferations, and AIL. We describe a case of polymorphic reticulosis (lethal midline granuloma) arising in an HIV-infected individual, who later progressed to AIDS, and review the literature on HIV-associated and EBV-associated T-cell neoplasia, LGL/T-cell proliferations, and AIL. The etiology of this AIL/T-cell NHL, especially in relation to EBV and HIV, is discussed.
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PMID:Angiocentric immunoproliferative lesion/T-cell non-Hodgkin's lymphoma and the acquired immune deficiency syndrome: a case report and review of the literature. 224 97

Multifocal retinochoroiditis was diagnosed in 16 patients (13 female, 3 male). The patients also had cells in the vitreous, and in some cases pronounced retinal vasculitis, cystoid macular edema and papilledema. The patients' ages ranged from 62 to 77 years. There was anterior segment involvement in 13 cases. One patient had subretinal neovascularization in both eyes. Fluorescein angiograms revealed leaks from the retinal vessels, papilledema, and macular edema. So far, the authors have not succeeded in classifying the changes described as belonging to a known disease entity. The findings most closely resembled a disease described by Dreyer and Gass in 1984 as "multifocal choroiditis and panuveitis". Similar changes were seen by Tiedemann in 1987, in patients with Epstein-Barr viral antibodies.
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PMID:[Retinal vasculitis with multifocal chorioretinitis]. 229 Feb 94

A 21-year-old woman had suffered from repeated vasculitis and panniculitis with fever and chills after following mosquito bites since age 7. These manifestations were confirmed with a direct mosquito bite test during her admission to another hospital at age 17. The patient presented to our hospital with progressive dyspnea, productive cough and intermittent fever for one week. In addition to the bilateral infiltrative lesions on chest roentgenography, hepatosplenomegaly with an abnormal liver function test, pancytopenia, and elevated IgE were also detected. Blood and sputum cultures grew no microorganisms. Epstein-Barr virus-IgM, Cytomegalovirus-IgM and Mycoplasma pneumonia antibodies were all negative. Bone marrow aspiration and biopsy revealed histiocytosis with hemophagocytosis. No atypical histiocyte was found. The patient was put on dexamethasone treatment with improvement. Unfortunately, symptoms relapsed two weeks later. A repeated bone marrow aspiration and biopsy revealed a picture similar to the previous one. Despite antibiotic administration and ventilator support, a rapidly deteriorated course terminated in the patient's death by respiratory failure. Mosquito bite allergies were reported to be associated with malignant histiocytosis in Japan. However, atypical histiocytes were not found in our case upon repeated bone marrow aspirations, biopsies and skin biopsies. Mature histiocytes with hemophagocytosis were prominent instead. Reactive histiocytosis was thus favored rather than malignant histiocytosis. Although opportunistic infection cannot be excluded, we propose that a mosquito bite allergy with consequent histiocytic activation by antigen, immune complex or IgE is the possible pathogenetic mechanism for hemophagocytic histiocytosis in this patient.
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PMID:[Mosquito bite allergies terminating as hemophagocytic histiocytosis: report of a case]. 257 73

Epstein-Barr virus (EBV), a ubiquitous human herpesvirus and an aetiological agent of infectious mononucleosis, has a unique tropism for B lymphocytes. Clinical and laboratory features of chronic active EBV infections are chronic or persistent infectious mononucleosis-like symptoms and high antibody titre against early antigens (EA). Kawasaki disease (KD), aetiology unknown, is thought to be self-limited immunologically mediated vasculitis. Clinical features of KD are fever, rash, mucositis, lymphadenopathy and coronary artery damage. We report here a child with chronic active EBV infection accompanied by dilatation of coronary arteries. All the EBV-determined nuclear antigen (EBNA)-positive lymphocytes had exclusively CD4 antigen, as revealed by dual staining immunofluorescence analysis. Southern blot hybridization showed that the purified CD4+ cells harboured EBV genome.
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PMID:Epstein-Barr virus genome-positive T lymphocytes in a boy with chronic active EBV infection associated with Kawasaki-like disease. 283 34

An 8-year-old maternally related relative of three boys who had developed agammaglobulinemia associated with Epstein-Barr virus (EBV)-induced infectious mononucleosis was studied for X-linked lymphoproliferative syndrome (XLP) in 1979. At that time, he demonstrated no striking immunologic aberrations and was seronegative for EBV. Subsequently, immunologic abnormalities including failure to switch from IgM to IgG antibody synthesis after secondary immunization with bacteriophage phi X174 were detected. In 1983, he experienced episodic intracerebral hemorrhages, with the second being fatal. At autopsy, necrotizing vasculitis and aneurysms involving arteries of the central nervous system were observed. Studies of blood obtained immediately before and after death failed to show antibodies to EBV. However, EBV genome was demonstrated in tissues obtained at autopsy by DNA hybridization studies. Fatal lymphoid vasculitis in this patient is unique among boys with XLP in the registry. These findings probably extend the phenotypic expressions of XLP.
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PMID:Necrotizing lymphoid vasculitis in X-linked lymphoproliferative syndrome. 298 73


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